Ectopic ACTH Syndrome Emerging 5 Years after the Diagnosis of Neuroendocrine Tumor
Ectopic ACTH syndrome (EAS) arising years after the diagnosis of a neuroendocrine tumor (NET) is exceedingly rare. We describe a case of EAS occurring five years after the diagnosis of a metastatic lung NET in a 61-year-old woman. She presented with severe hypokalemia but was not overtly Cushingoid...
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| Format: | Article |
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Wiley
2019-01-01
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| Series: | Case Reports in Endocrinology |
| Online Access: | http://dx.doi.org/10.1155/2019/6583467 |
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| author | Minghao Liu Diane Hamele-Bena John Ausiello Gabrielle Page-Wilson |
| author_facet | Minghao Liu Diane Hamele-Bena John Ausiello Gabrielle Page-Wilson |
| author_sort | Minghao Liu |
| collection | DOAJ |
| description | Ectopic ACTH syndrome (EAS) arising years after the diagnosis of a neuroendocrine tumor (NET) is exceedingly rare. We describe a case of EAS occurring five years after the diagnosis of a metastatic lung NET in a 61-year-old woman. She presented with severe hypokalemia but was not overtly Cushingoid on exam. Serum cortisol was 61mcg/dL after an overnight 1mg dexamethasone suppression test (<1.8mcg/dL) and urinary free cortisol was 7544 mcg/24h (<45mcg/24h), establishing the diagnosis of Cushing’s syndrome. Plasma levels of peptides which have been associated with EAS, Agouti-related peptide (AgRP) and the ACTH precursors POMC (31-kDa) and pro-ACTH (22-kDa), were elevated. Metyrapone was initiated, but hypercortisolism persisted and the patient succumbed to pneumonia shortly after presentation. Retrospective examination of biopsy tissues showed rare ACTH immunoreactivity at the time of initial diagnosis, followed by staining in a greater proportion of cells as the disease progressed, consistent with EAS arising years after the diagnosis of NET. Given the increase in mortality associated with EAS, this unusual case highlights the importance of early detection and raises the possibility that early immunohistochemical stains for ACTH and measurements of ACTH precursors may facilitate the identification of NETs at high risk for EAS. |
| format | Article |
| id | doaj-art-8b8c5b5bf5234192886d8a7e3feaf68e |
| institution | Kabale University |
| issn | 2090-6501 2090-651X |
| language | English |
| publishDate | 2019-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Case Reports in Endocrinology |
| spelling | doaj-art-8b8c5b5bf5234192886d8a7e3feaf68e2025-02-03T01:25:34ZengWileyCase Reports in Endocrinology2090-65012090-651X2019-01-01201910.1155/2019/65834676583467Ectopic ACTH Syndrome Emerging 5 Years after the Diagnosis of Neuroendocrine TumorMinghao Liu0Diane Hamele-Bena1John Ausiello2Gabrielle Page-Wilson3Endocrinology Division, Department of Medicine, Columbia University Medical Center, 650 West 168th St., Room 2012, New York, NY 10032, USADepartment of Pathology and Cell Biology, Columbia University Medical Center, Physicians and Surgeons Building, Room 404, 630 W 168th St., New York, NY 10032, USAEndocrinology Division, Department of Medicine, Columbia University Medical Center, Harkness Pavilion, 9th floor, 180 Fort Washington Ave., New York, NY 10032, USAEndocrinology Division, Department of Medicine, Columbia University Medical Center, 650 West 168th St., Room 2012, New York, NY 10032, USAEctopic ACTH syndrome (EAS) arising years after the diagnosis of a neuroendocrine tumor (NET) is exceedingly rare. We describe a case of EAS occurring five years after the diagnosis of a metastatic lung NET in a 61-year-old woman. She presented with severe hypokalemia but was not overtly Cushingoid on exam. Serum cortisol was 61mcg/dL after an overnight 1mg dexamethasone suppression test (<1.8mcg/dL) and urinary free cortisol was 7544 mcg/24h (<45mcg/24h), establishing the diagnosis of Cushing’s syndrome. Plasma levels of peptides which have been associated with EAS, Agouti-related peptide (AgRP) and the ACTH precursors POMC (31-kDa) and pro-ACTH (22-kDa), were elevated. Metyrapone was initiated, but hypercortisolism persisted and the patient succumbed to pneumonia shortly after presentation. Retrospective examination of biopsy tissues showed rare ACTH immunoreactivity at the time of initial diagnosis, followed by staining in a greater proportion of cells as the disease progressed, consistent with EAS arising years after the diagnosis of NET. Given the increase in mortality associated with EAS, this unusual case highlights the importance of early detection and raises the possibility that early immunohistochemical stains for ACTH and measurements of ACTH precursors may facilitate the identification of NETs at high risk for EAS.http://dx.doi.org/10.1155/2019/6583467 |
| spellingShingle | Minghao Liu Diane Hamele-Bena John Ausiello Gabrielle Page-Wilson Ectopic ACTH Syndrome Emerging 5 Years after the Diagnosis of Neuroendocrine Tumor Case Reports in Endocrinology |
| title | Ectopic ACTH Syndrome Emerging 5 Years after the Diagnosis of Neuroendocrine Tumor |
| title_full | Ectopic ACTH Syndrome Emerging 5 Years after the Diagnosis of Neuroendocrine Tumor |
| title_fullStr | Ectopic ACTH Syndrome Emerging 5 Years after the Diagnosis of Neuroendocrine Tumor |
| title_full_unstemmed | Ectopic ACTH Syndrome Emerging 5 Years after the Diagnosis of Neuroendocrine Tumor |
| title_short | Ectopic ACTH Syndrome Emerging 5 Years after the Diagnosis of Neuroendocrine Tumor |
| title_sort | ectopic acth syndrome emerging 5 years after the diagnosis of neuroendocrine tumor |
| url | http://dx.doi.org/10.1155/2019/6583467 |
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